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A sarcoma (malignant tumor) is a type of cancer that originates in the bones or muscles.
Soft tissue sarcomas are a rare form of cancer (malignant) that affects connective tissue such as muscles, tendons, ligaments, cartilage, synovial tissues, and the lining of blood vessels.
Several studies report that blood supply to soft tissue sarcomas is generally greater than that to adjacent normal tissues. Thus, the tumor receives blood supply, but its center is relatively underperfused compared with the tumor periphery.
Vascular sarcoma is a type of soft tissue sarcoma that potentially affects the lymphatic system and lining of the blood vessels anywhere in the body. It is most often found in children and adolescents.
Vascular sarcomas are subdivided into the following:
- Angiosarcoma: Most commonly affects the skin, liver, breast, and spleen tissue
- Hemangioendothelioma: Most commonly affects the lungs, liver, head, neck, intestines, musculoskeletal system, stomach, and lymph nodes
What are soft tissue sarcomas?
A sarcoma (malignant tumor) is a type of cancer that originates in the bones or muscles.
Two types of sarcomas include:
- Bone sarcoma: Such as osteosarcomas, Ewing family of tumors, and rhabdomyosarcoma
- Soft tissue sarcoma: Develops in connective tissue such as the fats, muscles, tendons, ligaments, nerves, fibrous tissues, blood vessels, or deep skin tissues
Soft tissue sarcomas can be found in any part of the body. Although most of them start in the arms or legs, they can be found in the trunk, head, and neck area; internal organs, and area in the back of the abdominal cavity.
13 types of soft tissue sarcomas
There are more than 50 types of soft tissue sarcomas, a few are very common and some of them are quite rare.
Sarcomas are classified based on the location of their origin:
- Leiomyosarcoma: Originates in the smooth involuntary muscles of the womb, digestive system, and blood vessels
- Gastrointestinal stromal tumor: Originates in the gastrointestinal tract
- Liposarcoma: Soft tissue sarcoma in fatty tissues or adipose tissues
- Malignant schwannoma or malignant peripheral nerve sheath tumors: Originate in the peripheral nervous system
- Angiosarcoma, hemangioendothelioma, hemangiopericytoma, and solitary fibrous tumor: Originates in the blood vessels
- Fibrosarcoma, dermatofibrosarcoma, low-grade fibromyxoid sarcoma, and fibromatosis: Sarcomas of the connective tissues
- Alveolar soft part sarcoma: Rare type that commonly starts in the legs
- Clear cell sarcoma: Originates in the tendons of the arms or legs
- Desmoplastic small round cell tumor: Rare sarcoma of the abdomen
- Epithelioid sarcoma: Originates in the tissues under the skin of the hands, forearms, feet, or lower legs
- Kaposi sarcoma: Originates in the cells lining the lymph or blood vessels
- Synovial sarcoma: Malignant tumor of the tissue around joints
- Undifferentiated pleomorphic sarcoma: Most often found in the arms or legs
6 risk factors for soft tissue sarcomas
Six risk factors for soft tissue sarcoma include:
- Genetic mutations
- Exposure to chemicals such as herbicides, arsenic, dioxin or thorium dioxide, and vinyl chloride
- Radiation exposure (history of radiation treatment for other types of cancer)
- Lymphedema in the arms or legs for a long time
- Human immunodeficiency virus and human herpesvirus 8 infections
- Certain inherited syndromes such as hereditary retinoblastoma, Li-Fraumeni syndrome, familial adenomatous polyposis, von Recklinghausen disease (neurofibromatosis), tuberous sclerosis, Gardner syndrome, Nevoid basal cell carcinoma syndrome, and Werner syndrome
Symptoms of soft tissue sarcomas
In the early stages, a soft tissue sarcoma may not cause any signs and symptoms.
As the tumor grows, it may present as:
- A noticeable painless lump or swelling under the skin, often on an arm or a leg
- Pressure on nearby organs, nerves, muscles, or blood vessels causing:
How is soft tissue sarcoma diagnosed?
Knowing the type of tumor helps predict the tumor response to specific treatments and allows to personalize the treatment.
To confirm the diagnosis, the doctor may recommend the following:
- Imaging tests: The area of concern can be evaluated by
- X-rays
- Computed tomography scans
- Magnetic resonance imaging
- Positron emission tomography
- Biopsy: A sample of tissue is taken from the body to examine it closely.
- Core needle biopsy: Samples from several sections of the tumor are obtained to be analyzed in the laboratory.
- Surgical or incisional biopsy: Surgical removal of a larger sample of tissue or removal of a small tumor entirely.
Is soft tissue sarcoma curable?
Depending on the size, type, location, and aggressiveness of the tumor, the treatment may comprise:
- Surgical removal: The most common treatment that involves removing cancer and some surrounding healthy tissue
- Radiation therapy: Involves treating cancer with high-powered beams of energy
- Chemotherapy: A drug treatment that uses chemicals to kill cancer cells
- Targeted drug therapy: For some cases of soft tissue sarcoma that have specific characteristics in their cells can be attacked by targeted drug treatments
